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  • Title: Autoimmune GFAP astrocytopathy presenting with remarkable CNS hyperexcitability and oculogyric crises.
    Author: Equiza J, Rodríguez-Antigüedad J, Campo-Caballero D, Iruzubieta P, Prada Á, Roncancio A, Fernández E, Ganzarain Oyarbide M, Arruti M, Urtasun MA, Castillo-Triviño T.
    Journal: J Neuroimmunol; 2021 Oct 15; 359():577695. PubMed ID: 34416409.
    Abstract:
    The autoimmune GFAP astrocytopathy has been associated with meningoencephalomyelitis that usually responds to glucocorticoids. We report a 20-year-old man that developed an acute and severe meningoencephalomyelitis with remarkable CNS hyperexcitability and oculogyric crises. CSF analysis showed hypoglycorrhachia, pleocytosis, elevated ADA, and CSF-immunofluorescence characteristic of autoimmune GFAP astrocytopathy. MRI showed lesions at thalamus, corpus-callosum, dorsal pons and dentate nucleus with associated myelitis. Immunotherapy led to a full recovery, although MRI activity was observed at follow-up. CNS hyperexcitability, typically seen in other immune-mediated syndromes, represents a novel presenting form to be included as part of the clinical spectrum of this entity.
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